Connective tissue diseases and vasculitis Flashcards
What are connective tissue diseases
Autoimmune rheumatic diseases
What is Systemic Lupus Erythematosus?
An inflammatory multisystem autoimmune disorder.
Who would most commonly present with SLE?
A female Afro-Caribbean of 20-40yrs
identify some factors related to the aetiology of SLE
genetics
oestrogen levels
immunological factors
environmental- UV light, smoking, Epstein-Barr Virus
Describe the pathogenesis of SLE
After apoptosis of cells cellular remnants remain called blebs which carry self antigens on their surface. These are normally cleared up but instead they travel to lymphoid tissue where they sensitise T-cells. This leads to the production of autoantibodies by B-cells, triggering of the complement system and abnormal cytokine production.
What characterises the pathology of SLE?
Deposition of IgG antibodies and complement proteins and influx of neutrophils + lymphocytes. Also vasculitis
There are many variant clinical manifestations of SLE. Joint involvement is the most common manifestation. What features may be shown
synovitis and joint tenderness 2+ joints
myalgia
Jaccoud’s arthropathy
How does SLE manifest in the skin?
characteristic butterfly rash erythema on face photosensitivity purpura urticaria alopecia Raynaud's phenomenon
As well as resulting in symptoms related to the joints and skin, what other ways may SLE present?
interstitial lung disease, pleural effusions pericarditis, endocarditis lupus nephritis depression, delirium, Psychosis, Sjorgen's syndrome
Main investigations for SLE?
blood test- FBC- leucopenia/thrombocytopenia/lymphopenia
autoantibodies- ANA, anti-dsDNA, anti-Ro, anti-Sm, anti-La
complements C3 and C4
treatment of SLE?
avoid excessive sun exposure
NSAIDs
hydroxychloroquine
corticosteroids
What is Primary Sjorgen’s syndrome? What are the main symptoms of Sjorgen’s?
The syndrome of dry eyes in the absence of RA and other autoimmune diseases
dry eyes, mouth, skin, vagina
fatigue
salivary and parotid gland enlargement
Which auto-antibodies are raised in Sjorgen’s?
anti-Ro, anti-La
Sjorgen’s increases risk of what serious condition?
Lymphoma
What are the two major characteristics of the pathology of Systemic Scleroderma (SSc)
Vasculopathy- widespread vascular damage
fibrosis- over lower dermis and internal organs (treatment symptom based or organ specific)
What is the main red flag feature of Systemic scleroderma?
Raynaud’s syndrome
What is the difference between LcSSc and DcSSc?
Limited and Diffuse cutaneous scleroderma differ in frequency 70%:30%, distribution (limited is restricted to hands, feet, face and forearms), and the clinical feature shown.
What are the main features of SSc?
Raynaud's Thickened skin- distribution varies- diffuse/limited? Tight skin other face, small mouth and beaky nose Telangiectasia GI trouble pulmonary hypertension pulmonary fibrosis myocardial fibrosis scleroderma renal crisis
Which are the two main autoantibodies shown in Scleroderma?
anti-centromeres
anti-topoisomerase
How is Raynaud’s syndrome treated?
hand warmers
vasodilators- Calcium channel blockers e.g. nifedipine
PDE-5 inhibitors
prostacyclins
What is the characteristic symptoms of the following conditions? Inflammatory myopathies, polymyalgia rheumatica and fibromyalgia
Myopathy- proximal muscle weakness
polymyalgia rheumatica- pain and stiffness
fibromyalgia- pain and fatigue
The aetiology of inflammatory myopathies are _________. they are more common in _________. they have an associated risk of ___________. When the skin is involved it is called ___________.
unknown
women
malignancy
dermatomyositis
What are the main clinical features of myalgia?
proximal muscle weakness
myalgia
difficulty with specific actions
insidious, over months or acute onset.
What three signs distinguish polymyositis from dermatomyositis?
Gottron’s sign (purple discolouration of knuckles)
shawl sign
heliotrope rash (purple discolouration around eyes)
How may inflammatory myopathies effect other organs?
interstitial lung disease dysphagia myocarditis polyarthritis Raynaud's
What may be identified in the blood tests of myositis patients? what other investigations would you do?
Raised CK, ANA, Myositis-specific antibodies, others
Electromyography
needle muscle biopsy
MRI
Two main treatments of inflammatory myopathies?
corticosteroids and immunosuppression
Which age group most commonly suffer from polymyalgia rheumatica?
almost exclusively over 50s
What symptoms characterise polymyalgia rheumatica?
Pain and stiffness of shoulders and hip- worse in the morning
fever, fatigue weight loss
What is giant cell arteritis also known as?
temporal arteritis
What is giant cell arteritis?
It is an inflammatory granulomatous arteritis of large cerebral arteries which occurs in association with PMR
What are the signs/symptoms of giant cell arteritis?
severe headaches tenderness and swelling of temporal arteries jaw claudication pain on the scalp loss of sight in one eye
What is the definitive test for PMR and GCA? What other test can be used?
temporal artery biopsy
temporal artery USS
What are the starting daily doses of prednisolone for PMR and GCA?
PMR- 15mg
GCA- 40-60mg
afterwards is gradually reduced
______________ is the most common cause of chronic musculoskeletal pain in women aged 20-50yrs
Fibromyalgia
What are the major symptoms of fibromyalgia?
Chronic widespread pain
Fatigue- disrupted and unrefreshing sleep
tender points detectable on touch
identify three pharmo treatments of FM and one non-pharmo treatment
Anti-depressants, gabapentin, pregabalin, analgesics
cognitive behavioural therapy
Define vasculitis
Inflammation of blood vessel walls
What are the two main features used in defining vasculitides?
size of the vessel- Large-vessel, medium-vessel, small-vessel
presence/absence of ANCA- Anti-neutrophil cytoplasmic antibodies
What systemic symptoms are common to all vasculitides?
fever, malaise, fatigue, weight loss
identify three similarities between Takuyasu’s arteritis and Giant cell arteritis
both large cell vasculitides
both effect aorta and its major branches
both show granulomatous inflammation
How is vasculitis normally treated?
with corticosteroids and/or immunosuppressant drugs
Give three examples of ANCA positive small cell vasculitides
granulomatosis with polyangiitis (GPA)
eosinophilic granulomatosis with polyangiitis (EGPA)
Microscopic polyangiitis (MPA)
Give one example of an ANCA negative vasculitis?
Henoch-Schonlein Purpura (HSP)
